Peripheral Exudative Hemorrhagic Chorioretinopathy (PEHCR): Diagnostic and Therapeutic Challenges.

Background and Objectives: Peripheral exudative hemorrhagic chorioretinopathy (PEHCR) is a peripheral retinal vascular abnormality that is likely underreported. We review the differential diagnoses, etiology, and treatment options for PEHCR. Methods: We present a case of an asymptomatic 72-year-old female referred following left eye fundus photography finding of the peripheral lesion. Results: Fundus photography demonstrated a large temporal pigment epithelial detachment (PED) with adjacent fibrovascular membrane. Optical coherence tomography (OCT) confirmed the PED with trace subretinal fluid. Fluorescein angiography (FA) demonstrated early and late hypofluorescence of the PED with late leakage of the adjacent temporal fibrovascular membrane. Observation was elected, visual acuity remained unaffected, and the PED spontaneously resolved. Conclusions: Due to the peripheral location, patients often present as asymptomatic; however, vision loss can occur due to vitreous hemorrhage or extension of subretinal fluid, hemorrhage, or exudate to the macula. Commonly, these lesions are referred with concern for choroidal melanoma due to their large, dark, elevated presentation in the peripheral retina. Multimodal testing using B-scan, FA, and OCT is important in establishing the proper diagnosis. PEHCR lesions can often be observed without treatment, though intravitreal injection of anti-VEGF is increasingly used to prevent secondary causes of vision loss.

Coats' Disease in a Patient With Cornelia de Lange Syndrome: Management With Laser and Bevacizumab.

Cornelia de Lange syndrome is a congenital disorder with multisystem abnormalities including multiple ocular findings. The authors report a case of Coats' disease in a patient with Cornelia de Lange syndrome who was successfully treated with laser and intravitreal bevacizumab. This case demonstrates the importance of fluorescein angiography in making the diagnosis and directing treatment and the efficacy of combined laser with intravitreal anti-vascular endothelial growth factor therapy for persistent vascular leakage associated with Coats' disease in Cornelia de Lange syndrome. [J Pediatr Ophthalmol Strabismus. 2023;60(4):e45-e48.].

CHOROIDAL NEOVASCULARIZATION AND CHORIORETINAL ATROPHY IN A PATIENT WITH MELANOMA-ASSOCIATED RETINOPATHY AFTER IPILIMUMAB/NIVOLUMAB COMBINATION THERAPY.

PURPOSE: To describe a case of choroidal neovascularization (CNV) and chorioretinal scarring in a patient with melanoma-associated retinopathy after ipilimumab/nivolumab combination immune therapy for malignant melanoma. METHODS: Retrospective case report with fundus photography, fluorescein angiography, optical coherence tomography, and electroretinography. RESULTS: A 65-year-old woman presented with symptoms of photopsia and visual field loss. She had previously undergone ipilimumab/nivolumab combination chemotherapy treatment for malignant melanoma 14 months earlier coinciding with the onset of her visual symptoms. Fundus photography showed bilateral atrophic chorioretinal lesions and peripheral retinal pigment epithelial changes. Fluorescein angiography revealed retinovascular leakage in both eyes with CNV in the right eye. Optical coherence tomography showed a pigment epithelial detachment with subretinal fluid and subretinal hyperreflective material consistent with occult CNV. Visual field testing showed generalized visual field loss in both eyes. Bloodwork discovered an elevated angiotensin-converting enzyme. Electroretinography revealed abnormal peripheral rod and cone function with impairment of the photoreceptor and inner nuclear layer. Serum Western blot was positive for 60 kDa antiretinal autoantibody. After a single bevacizumab injection in the right eye, CNV resolved and visual acuity improved from 20/50 before the injection to 20/25 3 months after the injection. Visual acuity in the left eye deteriorated for months to counting fingers but then improved to 20/100 on follow-up examinations. CONCLUSION: Ipilimumab and nivolumab have been associated with immune-related ocular adverse effects. We report a case of combination therapy presenting with chorioretinal scarring and subsequent CNV in a patient with melanoma-associated retinopathy, a rare yet important adverse effect.

Ocular Ischemic Syndrome With Congenital Absence of Internal Carotid Artery.

Purpose: This article describes a case of ocular ischemic syndrome (OIS) in a patient with a congenitally absent left internal carotid artery (ICA). Methods: Retrospective case report with anterior-segment and fundus photography, fluorescein angiography (FA), and computerized tomography angiography (CT-A). Results: A 31-year-old-man was found to have neovascularization of the iris (NVI) and angle of the left eye. FA showed capillary nonperfusion in the temporal periphery. He required intravitreal bevacizumab and triamcinolone injections and 2 panretinal photocoagulation treatments for persistent rubeosis and cystoid macular edema. Transient right-eye vision loss prompted CT-A, revealing an absent left ICA. Three years following presentation, FA continued to show delayed arteriovenous flow suggestive of OIS. He has required intravitreal bevacizumab injections every 12 weeks for persistent NVI. Conclusion: Congenitally absent left ICA with resultant cerebrovascular insufficiency is a rare cause of OIS, underscoring the pathophysiological principles of insufficient blood supply to the ophthalmic artery.